Hostname: page-component-76fb5796d-2lccl Total loading time: 0 Render date: 2024-04-25T08:24:01.953Z Has data issue: false hasContentIssue false
  • English
  • Français

Bilateral profound sudden sensorineural hearing loss presenting a diagnostic conundrum in a child with sickle cell anaemia

Published online by Cambridge University Press:  17 September 2008

A D Mace ,
M S Ferguson ,
M Offer ,
K Ghufoor  and
M J Wareing
A D Mace*
Affiliation:
Department of Otolaryngology, St Bartholomew's and The Royal London Hospitals NHS Trust, London, UK
M S Ferguson
Affiliation:
Department of Otolaryngology, St Bartholomew's and The Royal London Hospitals NHS Trust, London, UK
M Offer
Affiliation:
Department of Haematology, St George's Healthcare NHS Trust, London, UK
K Ghufoor
Affiliation:
Department of Otolaryngology, St Bartholomew's and The Royal London Hospitals NHS Trust, London, UK
M J Wareing
Affiliation:
Department of Otolaryngology, St Bartholomew's and The Royal London Hospitals NHS Trust, London, UK
*
Address for correspondence: Mr Alasdair Mace, 2 Whitelands Avenue, Chorleywood WD3 5RD, UK. E-mail: alasdairmace@hotmail.com
Get access Rights & Permissions [Opens in a new window]

Abstract

Objective:

To present the first published case of a child with bilateral profound sudden sensorineural hearing loss found in association with sickle cell anaemia, and to demonstrate the importance of early recognition, investigation and empirical treatment of sudden sensorineural hearing loss.

Method:

Case report and review of world literature.

Case report:

The authors present the case of a seven-year-old child with known sickle cell anaemia, who presented with bilateral profound sensorineural hearing loss developing over a period of five days. There was a history of ophthalmological disease in the preceding weeks, and inflammatory markers were raised. The differential diagnosis included a vaso-occlusive or inflammatory aetiology such as Cogan's syndrome, and treatment for both was instigated. Hearing thresholds did not recover, and the patient underwent cochlear implantation 12 weeks later.

Conclusion:

Sudden sensorineural hearing loss has a variable aetiology and is rare in children. Immediate treatment for all possible aetiologies is essential, along with targeted investigations and early referral for cochlear implantation if no recovery is demonstrated.

Keywords

Sickle Cell AnaemiaSensorineural Hearing LossCogan's SyndromeChild
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 123 , Issue 7 , July 2009 , pp. 811 - 816
Copyright
Copyright © JLO (1984) Limited 2008

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1 Ajulo, SO, Osiname, AI, Myatt, HM. Sensorineural hearing loss in sickle cell anaemia – a United Kingdom study. J Laryngol Otol 1993;107:790–4CrossRefGoogle ScholarPubMed
2 Mgbor, N, Emodi, I. Sensorineural hearing loss in Nigerian children with sickle cell disease. Int J Pediatr Otorhinolaryngol 2004;68:1413–16CrossRefGoogle ScholarPubMed
3 Jovanovic-Bateman, L, Hedreville, R. Sensorineural hearing loss with brainstem auditory evoked responses changes in homozygote and heterozygote sickle cell patients in Guadeloupe. J Laryngol Otol 2006;120:627–30CrossRefGoogle ScholarPubMed
4 Whitehead, RE, MacDonald, CB, Melhem, ER, McMahon, L. Spontaneous labyrinthine hemorrhage in sickle cell disease. Am J Neuroradiol 1998;19:1437–40Google ScholarPubMed
5 Tavin, ME, Rubin, JS, Camacho, FJ. Sudden sensorineural hearing loss in haemoglobin SC disease. J Laryngol Otol 1993;107:831–3CrossRefGoogle ScholarPubMed
6 Hotaling, AJ, Hillstrom, RP, Bazell, C. Sickle cell crisis and sensorineural hearing loss: case report and discussion. Int J Pediatr Otorhinolaryngol 1989;17:207–11CrossRefGoogle Scholar
7 O'Keeffe, LJ, Maw, AR. Sudden total deafness in sickle cell disease. J Laryngol Otol 1991;105:653–5CrossRefGoogle ScholarPubMed
8 Zadeh, MH, Storper, IS, Spitzer, JB. Diagnosis and treatment of sudden-onset sensorineural hearing loss: a study of 51 patients. Otolaryngol Head Neck Surg 2003;128:92–8CrossRefGoogle ScholarPubMed
9 Hughes, GB, Freedman, MA, Haberkamp, TJ, Guay, ME. Sudden sensorineural hearing loss. Otolaryngol Clin North Am 1996;29:394–405CrossRefGoogle ScholarPubMed
10 Byl, FM Jr. Sudden hearing loss: eight years' experience and suggested prognostic table. Laryngoscope 1984;94:647–61CrossRefGoogle ScholarPubMed
11 Mattox, DE, Simmons, FB. Natural history of sudden sensorineural hearing loss. Ann Otol Rhinol Laryngol 1977;86:463–80CrossRefGoogle ScholarPubMed
12 Shikowitz, MJ. Sudden sensorineural hearing loss. Med Clin North Am 1991;75:1239–50CrossRefGoogle ScholarPubMed
13 Cole, RR, Jahrsdoerfer, RA. Sudden hearing loss: an update. Am J Otol 1988;9:211–15Google ScholarPubMed
14 Perlman, HB, Kimura, R. Experimental obstruction of venous drainage and arterial supply of the inner ear. Ann Otol Rhinol Laryngol 1957;66:537–46CrossRefGoogle ScholarPubMed
15 Suga, F, Preston, J, Snow, JB Jr. Experimental microembolization of cochlear vessels. Arch Otolaryngol 1970;92:213–20CrossRefGoogle ScholarPubMed
16 Belal, A Jr. Pathology of vascular sensorineural hearing impairment. Laryngoscope 1980;90:1831–9CrossRefGoogle ScholarPubMed
17 Cogan, DG. Syndrome of nonsyphilitic interstitial keratitis and vestiboloauditory symptoms. Arch Ophthalmol 1945;33:44–9CrossRefGoogle Scholar
18 Bennett, FM. Bilateral recurrent episcleritis associated with posterior corneal changes, vestibulo-auditory symptoms and rheumatoid arthritis. Am J Ophthalmol 1963;55:815–18CrossRefGoogle ScholarPubMed
19 Conlin, AE, Parnes, LS. Treatment of sudden sensorineural hearing loss. A systematic review. Arch Otolaryngol Head Neck Surg 2007;133:573–81CrossRefGoogle ScholarPubMed